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门-窦血管病——门静脉高压症的一种新型疾病谱

李玉林 符艳秋 周丽诗 李雨芙 羊东晔

引用本文:
Citation:

门-窦血管病——门静脉高压症的一种新型疾病谱

DOI: 10.3969/j.issn.1001-5256.2022.12.032
基金项目: 

2018年深圳市科技计划基础研究(学科布局)项目 JCY20180508153013853

利益冲突声明:所有作者均声明不存在利益冲突。
作者贡献声明:李玉林负责检索文献及撰写文章;符艳秋、周丽诗、李雨芙负责分析和总结文献;羊东晔负责提出思路,指导写作及修改文章。
详细信息
    通信作者:

    羊东晔,yangdy@hku-szh.org

Porto-sinusoidal vascular disease——A novel entity of portal hypertension

Research funding: 

Shenzhen Science and Technology Innovation Commission Fundamental Research Key Projects of 2018 JCY20180508153013853

More Information
  • 摘要: 门-窦血管病(PSVD)是一种以门静脉高压症为主要临床特征,但无肝硬化,或无肝硬化相关危险因素,且影像学检查门静脉、肝静脉及下腔静脉通畅,需要活检病理诊断的一种疾病。该疾病在东西方的发病率存在很大差异;已知与自身免疫、血液病及血栓前疾病、感染、毒物或药物暴露、遗传、代谢相关,但确切的病因仍然未知。该疾病有三种典型组织病理学改变,分别是闭塞性门静脉病、结节性再生增生、不完全性间隔纤维化。PSVD早期临床表现没有特异性,晚期与肝硬化相似,影像上主要表现为门静脉高压相关并发症的征象,但肝脏硬度正常或稍增加,诊断需要肝活检;治疗上参照肝硬化的标准;该疾病预后明显优于肝硬化。该综述回顾性总结PSVD的流行病学、发病机制、临床诊疗等,以期提高临床医生对该病的认识。

     

  • 表  1  PSVD组织学特征

    Table  1.   Histological features of porto-sinusoidal vascular disease

    直接证据 间接证据
    闭塞性门静脉病变
    结节性再生增生
    不完全性间隔纤维化
    肝窦扩张,巨窦
    窦周纤维化
    中央静脉异常
    门静脉疝,门静脉周围异常血管,门静脉残余,门静脉多样性等
      注:(1)组织学特征满足直接证据之一者就可诊断PSVD;(2)组织学特征满足一项或多项间接证据需怀疑PSVD。
    下载: 导出CSV

    表  2  PSVD诊断标准

    Table  2.   Diagnostic criteria for porto-sinusoidal vascular disease

    肝活检(长度≥20 mm或≥10门静脉束或病理学家认为足够)排除肝硬化且排除其他原因PH
    (1)满足任意一条典型临床表现:①胃食管或异位静脉曲张;②门静脉高压性出血;③影像学可见侧支循环形成
    (2)满足任意一条典型组织学特征:①闭塞性门静脉病;②结节性再生增生;③不完全性间隔纤维化
    (3)任意一条非典型临床表现:①脾肿大;②腹水;③血小板减少+任意一条非典型组织学特征:a.门静脉通道异常(动脉增殖、扩张等);b.结构异常(门静脉束和中央静脉分布不规则);c.肝窦扩张;d.轻度窦周纤维化
    下载: 导出CSV
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  • 收稿日期:  2022-05-15
  • 录用日期:  2022-06-20
  • 出版日期:  2022-12-20
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